Case Report: Refractory childhood-onset takayasu arteritis with digital clubbing and extensive supra-aortic and lower-limb arterial disease

Childhood-onset Takayasu arteritis is rare, and its early picture is often too vague to point at a large-vessel vasculitis. We report a 12-year-old girl who presented with 13 days of recurrent severe retrosternal chest pain and diffuse colicky abdominal pain, on a background of six months of exertional fatigue and painful swelling of the right fingertips, without fever, weight loss or night sweats. Examination showed bilateral digital clubbing, a right arm blood pressure below the 5th percentile with left arm and lower limb readings between the 50th and 90th percentiles, an absent right radial pulse, and weak brachial and right posterior tibial pulses. The erythrocyte sedimentation rate was 110 mm/h and C-reactive protein 82 to 93 mg/L. Computed tomography angiography showed mild concentric mural thickening of the aortic arch with severe stenosis of the right common and internal carotid arteries, near-occlusion of the right subclavian artery, moderate to severe brachiocephalic stenosis, moderate narrowing of the left common carotid and left subclavian arteries, and moderate stenosis of the right common and superficial femoral arteries. The abdominal aorta and its visceral branches were spared. She met the EULAR/PRINTO/PRES (Ankara 2008) classification criteria for childhood Takayasu arteritis, and the arterial distribution corresponds to Numano type IIa, together with peripheral lower-limb involvement that the Numano scheme does not cover. Disease progressed on intravenous methylprednisolone, oral prednisolone, methotrexate, aspirin and low-molecular-weight heparin, culminating in an 80% stenosis of the right common femoral artery. Adalimumab was started and later replaced by tocilizumab, after which the femoral peri-arterial inflammatory changes resolved on imaging and acute-phase reactants normalized. Blood pressure asymmetry, pulse deficits and the established stenoses persisted. This case suggests that normal inflammatory markers in childhood-onset Takayasu arteritis can coexist with fixed arterial damage, and that judgements about disease activity are better based on clinical findings and serial imaging together than on acute-phase reactants alone.